Systemic amyloidosis by Cardiovascular Magnetic Resonance

• Main Author: Fontana, M.
• Other Authors: Moon, J. C. ; Hawkins, P. N.
• Published: University College London (University of London) 2016
• Online Access: https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.746199

Systemic amyloidosis is an infiltrative disorder caused by amyloid deposition in the extracellular space. Two main types of systemic amyloidosis affect the ventricular myocardium, immunoglobulin light chain (AL) and transthyretin (ATTR). These have different natural histories and prognosis but in both, cardiac involvement is the main driver of outcome. For cardiac amyloidosis, Cardiovascular Magnetic Resonance (CMR) with the late gadolinium enhancement (LGE) technique provides sensitivity for early detection but is highly dependent on operator skills and not quantitative - there is no current method of measuring cardiac amyloid burden. A new technique, T1 mapping permits tissue abnormalities to be directly visualised in a simple scan – the colour changes being instantly recognisable, either before contrast (native T1 mapping) or after, when the myocardial extracellular volume (ECV) can be measured. Furthermore, a widely available LGE approach, phase sensitive inversion recovery (PSIR) LGE, being less operator dependent, had potential for improved performance.